Teaching Case

A 16-year-old adolescent was referred to our institution due to recurrent urinary tract infections, difficulty starting urination, and sensation of incomplete bladder emptying for the past year. The patient had a history of malaria and frequent urinary tract infections (about 3 episodes per year).

Ultrasound revealed bilateral ureterohydronephrosis with slight atrophy of both kidneys’ parenchyma, as well as a diffusely thickened bladder wall and significant postvoid residue (Figure 1).

Renal scintigraphy confirmed bilateral renal dysfunction.

Uretrocystography showed vesical dysmorphia, with lobulated contours and reduced bladder compliance (Figure 2). There was difficulty in the progression of contrast beyond the proximal urethra.

Magnetic Resonance (MR) showed diffuse bladder wall thickening, with significant submucosa oedema, which had a fringed appearance and was more pronounced at the level of the bladder neck and trigone (Figure 3). Dilatation of the distal ureters was present (Figure 3).

Cystoscopy demonstrated a trabeculated bladder, with digitiform formations below the trigone vesical extending to the posterior urethra. The remaining mucosa showed signs of diffuse inflammation.

Biopsies were performed, and the result revealed nests of Von Brunn, cystitis cystica and glandularis foci, and intestinal metaplasia without dysplasia. There were no signs of neoplasia.

Cystitis cystica and cystitis glandularis are chronic reactive inflammatory disorders that occur in the setting of repeated irritation, such as infection, calculi, outlet obstruction, or even tumours [1]. Those conditions involve urothelium metaplasia, with formation of hyperplastic urothelial cell clusters called Brunn nests, which can lead to the development of fluid-filled pseudocystic structures in cystitis cystica or intestinal columnar mucin-secreting glands in cystitis glandularis [2,3]. Both conditions are usually present, rather than in their pure form, which can be called cystitis cystica et glandularis. Atypia and muscle invasion are not features and should suggest the diagnosis of carcinoma [1]. There is controversy about the potential premalignant nature of urothelial intestinal metaplasia [3].

It can occur at any age, with slight male predominance [4]. Symptoms include those of chronic irritation, such as frequency, dysuria, urgency, and hematuria [1].

Cystoscopy can show the bladder surface as rough and bumpy, resembling a cobblestone pattern. It can progress into a polypoidal mass, particularly in the bladder neck, and trigone areas, that can be mistaken for cancer [1].

Radiologically, cystitis cystica and glandularis may appear as diffuse bladder wall thickening or as polypoidal mass-like lesions within the bladder [4]. Ultrasound can assess bladder wall thickness, detect papillary/nodular projections, and evaluate urinary retention, incomplete bladder emptying and hydronephrosis [2]. Computerized tomography can also be useful in bladder evaluation, and to exclude mimickers. In the excretory phase, it can appear as multiple small-sized filling defects in the bladder wall [1]. Conventional cystograms may also provide a detailed look at the bladder and detect the presence of vesicoureteral reflux, which can coexist with or mimic the symptoms, especially in children [5].

MR provides a more detailed assessment of the urinary system. The bladder wall shows hypointensity in T1-weighted sequences. In T2-weight sequences, the signal intensity is variable, as it can be hyperintense due to oedema [1]. The muscle layer should be intact, and Wang et al demonstrated that cystitis glandularis shows absence or minimal restricted diffusion. These two features may be valuable to differentiate it from malignancy [6].

The treatment consists of removing the source of irritation and surgical excision of the area of inflammation or cystectomy in rare severe cases [1]. These patients should be monitored because of the possible association with malignancy [3,4].

Written informed patient consent for publication has been obtained.